Occult Diffuse Neoplasm in the Lungs: Intravascular Large B-Cell Lymphoma

Area of Science:

• Oncology
• Pulmonary Medicine
• Hematology

Background:

• Intrathoracic lymphomas commonly present with lymphadenopathy and lung abnormalities.
• Intravascular large B-cell lymphoma (IVLBCL) is a rare extranodal lymphoma subtype affecting small blood vessels, posing diagnostic challenges.

Purpose of the Study:

• To characterize the clinical presentation, diagnostic findings, and outcomes of patients with histopathologically proven IVLBCL in the lungs.

Main Methods:

• A computer-assisted search identified 5 patients with IVLBCL in the lungs at Mayo Clinic (2001-2018).
• Review of medical records, imaging studies (chest radiography, CT, PET), pulmonary function tests, echocardiography, and pathologic specimens.

Main Results:

• Five patients (median age 68, 4 male) were diagnosed with IVLBCL via lung biopsy or autopsy.
• All patients presented with dyspnea and systemic symptoms; imaging studies were largely unrevealing.
• Pulmonary function tests showed reduced diffusing capacity; 2/4 patients had pulmonary hypertension. One patient survived 4 years after chemotherapy.

Conclusions:

• IVLBCL diagnosis is challenging due to variable and nonspecific clinical and imaging findings.
• Consider IVLBCL in unexplained progressive dyspnea with systemic symptoms, even with normal imaging.
• Early diagnosis and treatment are critical for improving outcomes in IVLBCL.